WS04.3 Deficiency of dehydroepiandrosterone sulfate in pediatric and adult patients with cystic fibrosis

Objectives Dehydroepiandrosterone (DHEA) is the most abundant adrenal steroid, and the serum concentrations of its sulfate ester, DHEA-S, are 20-fold higher than those of any other circulating steroid hormone. Decreased levels of DHEA-S are associated with pulmonary hypertension and cardiovascular diseases, immune abnormalities, osteoporosis, glucose intolerance and diabetes, deterioration of lipid metabolism. The aim of the study was to evaluate DHEA-S concentrations in children and adults with cystic fibrosis (CF). Methods 125 pediatric (6–17 years) and 38 adult patients (18–28 years) with CF were enrolled into the study. Plasma DHEA-S and ACTH levels were assayed with commercially available kits. Results DHEA-S concentrations were significantly below the age-adjusted median values in 68% of children and 73% of adults with CF. The hormone deficiency was more pronounced in subjects with cirrhosis and in the group of young women where, respectively, 100% and 82% of participants had DHEA-S concentrations below the age-adjusted median values. Pediatric patients (6–11 years) with minimal DHEA-S levels demonstrated significantly lower Body Mass Indexes compared to those of CF children who had normal hormone concentrations (14.4 vs 16.3 kg/m 2 p Conclusion Our data indicate that most pediatric and adult CF patients suffer from DHEA-S deficiency. Since DHEA is a well-tolerated and inexpensive drug, its supplementation might prove useful for the clinical management of CF patients with low levels of the hormone.